PRMER MMN YETMEZLKLERDE TEHSTEN TEDAVYE

PRMER MMN YETMEZLKLER GZDEN KAIRMAMAK N NE YAPMALIYIZ?

18.12.2012

DR.TUE KALIN

PROF. DR. AYDAN KNCOULLARI

ATF OCUKATF

Sunum Plan

mmnite ve primer immn yetmezliin tanmlar ;epidemiyolojisi ve snflandrmas

Klinik bulgular ve tansal testler

PY tansnda 1. basamak hekiminin dikkat etmesi gereken noktalar

Kliniimizden vaka rnekleri

Hastaln tansnda yaanan sorunlar ve amann yollar

mmnite-Baklk

Konan kendisinden farkl yapya sahip maddeleri yabanc olarak alglayp onu yok etmek zere harekete gemesine immnite-baklk ad verilmektedir

Konak Savunmasnn nemli eleri

Doal baklkKazanlm baklk

Anatomik

Hcresel elemanlar

znebilenfaktrler

Hcresel baklkHumoral baklk

Deri

Mukoza

Mukosilier engel mekanizmas

Vcut svlarndaki koruyucu faktrler

Makrofaj

PNL

(ntrofil)

NK hcre

Kompleman

Akut faz proteinleri

Sitokinler

T lenfosit

B lenfosit

Antikor

Bebek ve ocuklarda mmn Sistem

Bebek ve ocuklarda immn sistemin baz farkllklar vardr

Salkl yenidoanlarda IgA ve IgM dzeyleri yabanc antijenle karlamamas nedeni ile dktr

zellikle son trimesterda plesental gei nedeniyle IgG, annedeki dzeyi yanstr.

Yenidoan dneminde T ve B lenfositlerin etkileimi ile difteri, tetanoz toksoidi gibi protein antijenlere kar antikor oluabilirken, T hcre bamsz polisakkarid antijenlere iki yana kadar yeterli yant geliememektedir

zetle, yenidoan dneminden balayarak hem humoral hem de hcresel immnitede sren bir olgunlama sz konusudur

Primer mmn Yetmezlik Nedir?

Baklk sisteminin bir ya da daha fazla hcresi, hcre ligand, plazma proteinleri, sitokin ve sitokin reseptr, hcre ii uyar sistemleri iin gerekli proteinlerin eksiklii ile meydana gelen, enfeksiyonlara eilimin artt, kaltsal bir grup hastal kapsar

Primer immn yetmezlikler;

Tekrarlayan enfeksiyonlar

Otoimmnite

Alerji

Malignitelere

yatknl arttran, bazen de sendromlarla karmza kan heterojenik bir dizi hastalktan oluur

1952 de Bruton un X e bal agammaglobunemiyi tanmlamasndan bugne teknolojideki gelimelere bal olarak 200 den fazla primer immn yetersizlik tanmlanmtr ve bunlardan yaklak 150 kadarnn molekler yaps tespit edilmitir

Amerika2.4/100.000 (1976-1980)

10.3/100.000 (2001-2006)

Savides C, Shaker M. Curr Opin Pediatr 2010; 22: 647-654

Fransa5/100.000

16.2/100.000

Savides C, Shaker M. Curr Opin Pediatr 2010; 22: 647-654

Trkiye30.5/100.000

Kl S ve ark. Clin Immunol 2012

16/100.000

Reisli I, AID Kongresi, 2012, Antalya

1.53/100.000

ESID, 2012

ESID

ESID(European Society for Immunodeficiencies) hasta kayt sistemi, PY hastalarnn klinik ve laboratuar verilerini derleyen, internet tabanl hasta kayt sistemidir

Avrupadaki PY lkelere Dalm

map_of_europe.jpg

Snflama

Uluslararas mmnoloji Dernekleri Birlii hastal 8 ana gruba ve alt gruplara ayrmtr

Kombine T ve B hcre yetmezlikleri

Antikor eksiklikleri

yi tanmlanm immn yetmezlikler

mmndisreglasyon hastalklar

Fagositer sistem say ve/veya fonksiyon eksiklikleri

Doal immnitedeki defektler

Otoinflamatuvar hastalklar

Kompleman eksiklikleri

IUIS-PID-CC. Frontiers in Immunology 2011; 2: 1-26

ESID 2012

Primer immn yetmezlikler gerek klinik prezentasyon gerekse uygulanan tedavi bakmndan olduka heterojen bir gruptur

Asemptomatik Ciddi semptomatik

Tedavi gerektirmeyen Kemik lii Nakli

AgamaglobunemiAKY

Selektif Ig A Eksiklii

THI

Hedefimiz;

Erken tan ve doru tedavi ile hastaln seyrinin deiecei kritik hasta grubunu yakalamak

Bu hastalk grubunu tanmak iin bilinmesi gereken; klinikte,anamnezde ve tarama testlerindeki nemli noktalar aktarmak olacaktr

Ne Zaman mmn Yetmezlikten phelenmeli?

mmnolojik DeerlendirmeTarama TestleriFizik Muayeneyk

Primer mmn Yetmezlik in 10 Uyarc aret

10KlinBulgu210KlinBulgu210KlinBulgu210KlinBulgu210KlinBulgu210KlinBulgu2Ylda 4 otit

Ylda 2 sins infeksiyonu

2 ay antibiyotik kullanmYlda 2 pnmoniGelime geriliiTekrarlayan cilt veya organ abseleri10KlinBulgu210KlinBulgu210KlinBulgu210KlinBulgu2indexJM Trkiye (yeni)Persistan oral moniliazisIV tedavi gereksinimi2 derin doku infeksiyonu,Frsat m.o enfAile yks

Bu almada PY ngrmedeki 10 uyarc iaretin etkililii aratrlmtr

Aile yks

V antibiyotik kullanm

Tart alamama,kilo kayb

en belirleyici uyarc iaret olarak tespit edilmitir ve bu zelliklere sahip olup merkezlere refere edilen alma kapsamndaki hastalarn %96 snda PY tans konmutur

Subbarayan A,Clinical Features That Identify Children With Primary Immunodeficiency Diseases, Pediatrics. 2011;127:810-816

Primer mmn Yetmezlik Klinik Prezentasyonlar
Enfeksiyon

Primer immn yetmezlikli hastalarn hekime en sk bavuru nedenleri tekrarlayan enfeksiyonlardr

Ciddi primer immn yetmezlik vakalar, hayat tehdit eden enfeksiyonlarla karmza kabilir

3

TipiTekrarlayc

YeriOral moniliyazis,cilt mantar, diyare, akcier enfeksiyonu, cilt-derin doku absesi, sepsis

iddetiAr seyir, komplike gidi

SresiUzun sren, persistan

YantGe ve g

AjanAllmn dnda mikroorganizma ile enfeksiyon, frsat patojenler, virs, protozoa, mikobakteri

YatknlkSadece baz m.o.larla hastalk
(mikobakteri, salmonella, pnmokok, herpes gibi)

B hcresi bozukluklarPnomococcusH.nfluenzae,Streptococcus

T hcresi bozukluklarMantar,MikobacteriumVirsProtozoa

Fagositer ilev bozukluklarStaphylococcusPseudomonasEscherichia coliAspergillus

Kompleman BozukluklarGram(+) mikroorganizmalarNeisseria

Klinik Prezentasyon
A

Canl alar izleyen sistemik hastalk geliimi

BCG aktivasyonu

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

Klinik Prezentasyon;
Byme-Gelime Gerilii

yk Bizim Klavuzumuz
Aile yks

E akrabal

Aklanamayan erken ocukluk ya grubu

lmleri

Ailede olas immn yetersizlik yks

Akraba evliliinin yaygn olduu lkemizde, aile yks primer immn yetmezlik tansn koymada nemli bir ipucudur

Akraba Evlilii

Kilic SS ve ark., J Clin Immunol, 2012,145:368-372

PY hastalarnn %14.3 nde,akraba evlilii yks mevcuttur,ou da birinci derece kuzen evlilikleridir

kincioullar A, AID Kongresi, 2012, Antalya

AKY hastalarnn % 73 nde e akrabal mevcuttur

yk Bizim Klavuzumuz

nfant/erken ocukluk anda iddetli,nadir veya tekrarlayan enfeksiyon yks

Aile yks

-akraba evlilii

-karde lm

-PY yks

EVETAile hikayesine odaklanPY dn

HAYIR

Bakteriyel ya da fungal sepsis nedeni ile IV antibiyotik/antifungal kullanm yks?

Alama sonras durumunu sorgula

ocukta gelime gerilii/konjenital anomaliler de var m?PY dn

Subbarayan A,Clinical Features That Identify Children With Primary Immunodeficiency Diseases, Pediatrics 2011;127:810-817

PY Dier Klinik Prezentasyonlar

Otoimmnite

Sitopeniler

Hipotiroidi

nflamatuar Barsak Hastalklar

Malignite

Lenforetikler

Allerji

Egzema

Astm vb tablo

Dismorfik-Tanmlayc bulgular

Mikrosefali, Albinizm, Telenjiektazi, Ataksi

Anjiodem

Kanama diatezi

Trombositopeni

Kk trombositler

Agregasyon bozukluklar

Fizik Muayenede mmn Yetersizlik Dndrecek Bulgular

Yineleyen veya sreklilik gsteren ate

Dk persentil deerleri, byme erisinde duraklama, mikrosefali

Deri bulgular(mantar enf., dermatit, ar egzema, yaygn sebore, telenjiektaziler, parsiyel albinizm)

Lenfoid doku ve/veya tonsil yokluu

Lenfoid hiperplazi, hepatosplenomegali

Hastala spesifik tanmlayc bulgular

mmnolojik Deerlendirmede Tarama Testleri

Tam kan saym,PY

Ntropeni(